Severe lower tracheal stenosis in infancy.

16 infants with severe distal tracheal stenosis (TS) seen from 1978-1988 are reviewed. Diagnosis was established by endoscopy and confirmed by radiographic methods, including magnetic resonance imaging (MRI) in three cases. Types of TS ranged from segmental stenosis to complex lesions involving the carina and bronchi. Associated vascular anomalies were found in nine patients (56%). All patients needed long-term intubation or a tracheostomy (seven cases) for stenting of the airway. Patients with congenital TS (CTS) were treated by tracheal resection (two survivors), dilatation (one survivor, two non-survivors), or observation (two non-survivors). Patients with pulmonary artery sling (PAS) were treated by division of the vascular sling (three survivors, one non-survivor), or combined vascular and tracheal surgery (one non-survivor). One patient with dilated pulmonary arteries compressing both main bronchi died in spite of oxygen therapy. Two cases of compression by an anomalous aorta were managed with dilatation (one survivor, one non-survivor). One patient with a severe stricture after a tracheal resection was successfully treated by dilatation. The overall mortality was 50%. We conclude that 1. endoscopic examination of the tracheobronchial tree and thorough evaluation of associated anomalies is mandatory in symptomatic patients; 2. if indicated surgical correction is the treatment of choice for tracheal stenosis; 3. dilatation and stenting of the stenosis are invaluable tools in the management of these infants.